Serotonin-Secreting Neuroendocrine Tumours of the Pancreas.
pancreatic neuroendocrine neoplasm
primary pancreatic carcinoid
serotonin-producing pancreatic tumour
serotonin-secreting pancreatic tumour
Journal
Journal of clinical medicine
ISSN: 2077-0383
Titre abrégé: J Clin Med
Pays: Switzerland
ID NLM: 101606588
Informations de publication
Date de publication:
06 May 2020
06 May 2020
Historique:
received:
03
04
2020
revised:
01
05
2020
accepted:
02
05
2020
entrez:
10
5
2020
pubmed:
10
5
2020
medline:
10
5
2020
Statut:
epublish
Résumé
Serotonin-secreting pancreatic neuroendocrine tumours (5-HT-secreting pNETs) are very rare, and characterised by high urinary 5-hydroxyindole-acetic acid (5-HIAA) levels (or high serum 5-HT levels). Patients with 5-HT-secreting pancreatic neoplasms observed in our unit (1986-2015) were included. Diagnosis was based on urinary 5-HIAA or serum 5-HT levels. Seven patients were enrolled (4 M/3 F), with a median age of 64 (range 38-69) years. Two patients had a carcinoid syndrome. Serum 5-HT was elevated in four patients. Urinary 5-HIAA levels were positive in six patients. The median tumour size was 4.0 (range 2.5-10) cm. All patients showed liver metastases at diagnosis. None underwent resective surgery; lymph node/liver biopsies were taken. Six lesions were well-differentiated tumours and one a poorly differentiated carcinoma (Ki67 range 3.4-70%). All but one patient received chemotherapy. Four patients received somatostatin analogues; three patients underwent ablation of liver metastases. One patient is alive with disease 117 months after observation. All the others died from disease progression after a follow-up within 158 months. Primary 5-HT-secreting pNETs are mostly metastatic to the liver; patients are not amenable to resective surgery. Despite high 5-HIAA urinary levels, few patients present with carcinoid syndrome. A five-year survival rate of 42.9% may be achieved with multimodal treatment.
Sections du résumé
BACKGROUND
BACKGROUND
Serotonin-secreting pancreatic neuroendocrine tumours (5-HT-secreting pNETs) are very rare, and characterised by high urinary 5-hydroxyindole-acetic acid (5-HIAA) levels (or high serum 5-HT levels).
METHODS
METHODS
Patients with 5-HT-secreting pancreatic neoplasms observed in our unit (1986-2015) were included. Diagnosis was based on urinary 5-HIAA or serum 5-HT levels.
RESULTS
RESULTS
Seven patients were enrolled (4 M/3 F), with a median age of 64 (range 38-69) years. Two patients had a carcinoid syndrome. Serum 5-HT was elevated in four patients. Urinary 5-HIAA levels were positive in six patients. The median tumour size was 4.0 (range 2.5-10) cm. All patients showed liver metastases at diagnosis. None underwent resective surgery; lymph node/liver biopsies were taken. Six lesions were well-differentiated tumours and one a poorly differentiated carcinoma (Ki67 range 3.4-70%). All but one patient received chemotherapy. Four patients received somatostatin analogues; three patients underwent ablation of liver metastases. One patient is alive with disease 117 months after observation. All the others died from disease progression after a follow-up within 158 months.
CONCLUSIONS
CONCLUSIONS
Primary 5-HT-secreting pNETs are mostly metastatic to the liver; patients are not amenable to resective surgery. Despite high 5-HIAA urinary levels, few patients present with carcinoid syndrome. A five-year survival rate of 42.9% may be achieved with multimodal treatment.
Identifiants
pubmed: 32384679
pii: jcm9051363
doi: 10.3390/jcm9051363
pmc: PMC7291028
pii:
doi:
Types de publication
Journal Article
Langues
eng
Déclaration de conflit d'intérêts
The authors declare no conflict of interest.
Références
Arch Intern Med. 1967 Nov;120(5):575-80
pubmed: 4293348
Int J Pancreatol. 1998 Apr;23(2):153-64
pubmed: 9629513
Eur J Cancer. 1996 Jun;32A(7):1109-16
pubmed: 8758239
Diabetes. 2011 Dec;60(12):3208-16
pubmed: 22013016
Virchows Arch. 2006 Apr;448(4):394-8
pubmed: 16418841
N Engl J Med. 1993 Oct 7;329(15):1073-8
pubmed: 8371728
Clin Endocrinol (Oxf). 2017 Aug;87(2):165-170
pubmed: 28464233
Histopathology. 1981 Nov;5(6):599-613
pubmed: 6119287
J Gastrointest Surg. 2007 Nov;11(11):1460-7; discussion 1467-9
pubmed: 17846854
Pancreas. 2011 Aug;40(6):883-95
pubmed: 21705949
Eur J Endocrinol. 2016 Nov;175(5):361-6
pubmed: 27491374
Neuroendocrinology. 2016;103(2):153-71
pubmed: 26742109
Cancer. 2005 Sep 15;104(6):1180-7
pubmed: 16104045
Pancreatology. 2018 Oct;18(7):792-798
pubmed: 30115562
JAMA Oncol. 2017 Oct 1;3(10):1335-1342
pubmed: 28448665
Mod Pathol. 1991 Nov;4(6):727-32
pubmed: 1788265
Lancet. 1963 Feb 2;1(7275):238-9
pubmed: 14000847
Virchows Arch. 2006 Oct;449(4):395-401
pubmed: 16967267
J Clin Endocrinol Metab. 1990 Jun;70(6):1702-9
pubmed: 1693375
Cancer. 2003 Feb 15;97(4):934-59
pubmed: 12569593
Surgery. 1989 Jan;105(1):86-92
pubmed: 2463683